The University of Alabama at Birmingham (UAB) Recessive Polycystic Kidney Disease Core Center (DAB RPKDCC) will establish a UAB-based interdisciplinary center of excellence in PKD-related research, with specific emphasis on recessive PKD. The UAB RPKDCC builds on a core cadre of UAB investigators with established collaborations focused on investigations of human ARPKD and/or mouse models of recessive PKD. The Core Center expands to include outstanding investigators from UAB (Institutional Research Base), as well as multiple institutions in the US and Canada (Extended Research Base). The collective expertise of the assembled investigators has been organized into four thematic groups: 1) cilia-related biology, including calcium-mediated mechanotransduction pathways; 2) regulation of epithelial transport pathways; 3) signaling pathways that are critical in epithelial differentiation; and 4) matrix biology and fibrosis. These areas of emphasis incorporate the major mechanisms implicated in the pathogenesis of recessive PKD. The Core Center investigators will benefit from access to a set of five complementary Biomedical Research Cores that will integrate existing intellectual and technological resources of the University and provide a set of services/resources that will enable innovative investigations in the four thematic areas. The proposed biomedical cores are: 1) Core A - The ARPKD Clinical and Genetic Resource; 2) Core B - The Engineered Mouse Resource; 3) Core C - The Cellular Physiology Resource; 4) Core D - The Tissue Characterization and Immunoreagent Resource; and 5) Core E - The Proteomic Resource. Taken together, these Cores and the extraordinary cohort of investigators assembled for this Center will provide the breadth and depth of expertise that is critical for innovative and productive research in recessive PKD. With its Extended Research Base that includes both clinical and basic investigators, this Core Center model will accelerate the translation of new investigative insights toward new therapies for ARPKD patients.